This 48YO male gave a history of blindness in his right eye since birth. Vision was no light perception. The anterior chamber was shallow with a stalk of tissue radiating from the nerve to the back of his crystalline lens. The macula was hypoplastic. The left eye was completely normal.
Learning Points:
Persistent fetal vasculature (PFV) is a failure of regression of fetal vessels and a common cause of leukocoria and subsequent amblyopia. There is no identifiable genetic association.
Characteristically, the affected eye is microphthalmic with a shallow chamber, a varying degree of cataract, and retrolental and/or posterior fibrovascular membranes that can cause retinal traction.
If diagnosed earlier in life, PFV can be surgically treated with a combined lensectomy and vitrectomy.
We are observing our patient, and it is unclear why he has no light perception vision, given the relatively mild structural findings.
